Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation

Neurology. 2002 Sep 10;59(5):767-9. doi: 10.1212/wnl.59.5.767.


A patient with hereditary neuropathy presented with asymmetric distal weakness. On nerve biopsy, there was demyelination and onion-bulb formation, and molecular analysis revealed that the patient was heterozygous for an MPZ mutation. The patient improved with corticosteroid treatment.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Adult
  • Biopsy
  • Female
  • Hereditary Sensory and Motor Neuropathy / drug therapy*
  • Hereditary Sensory and Motor Neuropathy / genetics*
  • Hereditary Sensory and Motor Neuropathy / pathology
  • Humans
  • Myelin P0 Protein / genetics*
  • Point Mutation*
  • Sural Nerve / pathology


  • Adrenal Cortex Hormones
  • Myelin P0 Protein